Hot Seat #42: 17 year old girl who “passed out”
Posted on: September 1, 2014, by : Katie Donnelly MD
by Katie Donnelly, Children’s National
with Shilpa Patel, Children’s National
The Case
17 year old female who emigrated from El Salvador eight months ago who presents after an episode where she lost consciousness. She had been watching TV with her brother at the time. She states she remembers her vision greying out but denies palpitations, chest pain, diaphoresis or fevers. Her brother reports that she seemed to slump over. Her brother called for their father who was in the next room; when he arrived, the patient was not breathing and was blue around the lips. He gave her two rounds of chest compressions and rescue breaths and then the patient “woke up”. Father called EMS at that time and they were transported to the hospital. Patient was not post-ictal but stated that she could not see out of her left eye. That complaint has now resolved. She denies any drug or alcohol use, sexual activity, or recent excessive exertion.
ROS: Worsening headache over the past two months, otherwise unremarkable. The headaches have become more frequent and with stronger intensity but she has not missed school. They resolve with ibuprofen. She has some “blurry vision and difficulty focusing” when she has the headaches, but not without them.
IMM: up to date at time of immigration
FHx: No family history of seizure, fainting, sudden death, cardiac disease on paternal side. Father does not know maternal family history
PE: Vitals: T 36.8 HR 71 BP 108/67 O2 sat 100% on RA
Gen: Awake, alert, no acute distress
HEENT: normocephalic, atraumatic. PERRL, EOMI
CV: rate regular, no murmurs or gallops, normal peripheral perfusion
Lungs: Clear bilaterally
Abdomen: Soft, nontender, nondistended.
Neuro: CN II-XII intact bilaterally. Vision grossly normal but acuity not performed. Normal fundocscopic exam without papilledema. Normal strength and sensation bilateral upper and lower extremities.
Questions for you:
Interim Update:
Orthostatics: normal
EKG: normal sinus rhythm
CBC: WBC 5.6, Hgb 13.9, Hct 40.2, Plt 266
CMP: Na 138, K 4, Cl 103, HCO3 30, BUN 10, Cr 0.8, Gluc 93, Ca 9.2, Tot Prot 8.8 AST 18, ALT 19, tBili 0.6
Urine pregnancy negative
UTox, salicylate, acetaminophen NEG
You have just received these results when the resident comes to you and tells you that the patient is again complaining of vision loss in her left eye. You decide to obtain head imaging. Head CT : 4 mm well circumscribed calcification in the right occipital lobe surrounded by 1 cm of edematous changes.
How would you approach this case? Please share your opinions by clicking on “What do you think?” below.
Come back later for the denouement of this case.
sounds like neurocystercicosis. stay away from the piglets.
if there are calcifications, i think this implies that it’s been there a long time and she may not have active parasite but symptoms from the subsequent edema. if there is edema, than no LP from me. i would probably load with keppra to prevent seizures. even with a radiologic diagnosis treatment can be controversial because medical management can cause more local edema depending on the phase of parasite life cycle (i think). i guess the real question is whether the lesion is causing the eye symptoms or if there are other local complications that could be contributing to acute vision loss such as retinal artery occlusion or increased IOP.
so i would:
–admit to ICU
–tell them to consult neurosurgery and ID (for presumed cystercicosis)
–I would call ophthalmology for emergent exam
–ponder why the kid’s bicarb is 30
–do an exposure history (animals, herbals, medicines, etc)
–no hyperosmolar therapy unless there is AMS or global edema (it’s not always good for localized edema)
The presenting history of passing out, turning blue around the lips, requiring compressions and breaths to come to sounded like a cardiac etiology (i.e. a non-perfusing/fatal arrhythmia). But the unilateral vision loss that resolved by the time of arrival in the ED doesn’t quite fit with just a cardiac etiology. Instead of jumping first to the CT (especially with normal Neuro exam in the ED), I would flush out any possible cardiac etiology of the symptoms. EKG, CBC, BMP, Mg, Phos, Utox +/- CXR and Cardiology consult. But history of worsening HA and the brief unilateral vision loss is unusual for an arrhythmia and also unusual for a seizure. I’d be concerned about a TIA/CVA and therefore would want to get the best imaging possible to visualize the brain for a possible stroke… MRI (+/- MRA/MRV after discussing with neurology). That said, CT is the quickest to get so I voted for that to rule out badness quickly, then would follow it up with MRI as inpatient. Agree that calcified parenchymal lesion likely neuro cystercisosis. Also possible would be TB, metastatic or primary oncologic lesions in the brain. Still not sure how the solitary lesion’s location explains complete LOC requiring compressions though??? Definitely admit to PICU for neuro monitoring.
I concur with Dave Mathison, the description of the lesion and the presentation is really consistent with neurocysticercosis. However, I’d argue that she does not need an emergent ophthalmology consultation. All she needs instead is a good exam of her confrontational visual fields. My guess is that her complaint of loss of vision in her left eye is really a visual field cut (i.e.,loss of vision in her left visual field, or left homonymous hemianopsia), consistent with the head imaging findings of edema in the right occipital lobe. Here’s how I put it all together. The child has this neurocysticercosis lesion in the right occipital lobe that is causing edema and likely intermittent focal seizures with resultant left homonymous hemianopsia (loss of the left visual field). The event leading to presentation likely represented a secondary generalization of this initial focal seizure.
I agree with Dewesh, Jamil and Dave….this patient is presenting with neurocysticercosis (NCC).
The clinical presentation is classic. Our patient presents from an endemic region (Central/South America, sub-Saharan Africa, India or Asia) with a new seizure (non focal neuro exam and no other systemic illness) AND a CT brain showing a single enhancing lesion – making our diagnosis of NCC very likely.
Regarding the imaging and edema present, cyst degeneration usually occurs 3-5 years (can be up to 30 years) after exposure and that is what causes the local edema which then causes the seizures and in our case the vision changes. Although our imaging does not mention a cyst (probably because it is a non-contrast CT), cysts and calcifications can both be present at the same time and the cysts are the sites of active disease.
As Dewesh stated, the patient is probably misperceiving a left sided field loss in both eyes as loss of vision in only the left eye. It helps to look at Figure 2 in Sabah’s excellent UpToDate chapter on Acute Vision Loss in Children (link included below). Looking a Figure 2, we can see that a homonymous hemianopsia (same side visual field deficit in both eyes) results from a lesion behind the optic chiasm, or in our case a lesion in the visual cortex in the right occipital lobe (see #8 on Figure 2).
Dewesh’s rationale for the acute transient vision loss and preceding seizure that generalized is most likely what happened. However, ocular cysticercosis does exist and inflammation around degenerating cysticerci in the eye can threaten vision. Vision loss due to ocular inflammation would be unilateral (in the left eye – the same eye as the reported vision loss for our patient). This would support Dave’s recommendation for an urgent opthal eval. However ocular cysticercosis is rare and usually associated with the start of treatment. Either way she definitely needs a consult prior to treatment.
Finally to take some of the blame off the little piglets. Humans do not get neurocystircercosis from eating pork, they get an adult tapeworm infestation. These tapeworms can live in the intestine for several years and it is their eggs which when excreted in human feces infect other humans in environments with poor water sanitation. Food for thought. He..he..he.. ☺
You will have to be on the intranet for these to work, as you need access to UpToDate:
UpToDate – Acute Vision Loss in Children
http://www.uptodate.com/contents/approach-to-acute-vision-loss-in-children?topicKey=EM%2F6441&elapsedTimeMs=0&source=search_result&searchTerm=amaurosis+fugax&selectedTitle=2%7E31&view=print&displayedView=full#
Link to Figure 2 ( look at # 8) in the UpToDate Chapter: http://www.uptodate.com/contents/image?imageKey=NEURO%2F74068&topicKey=EM%2F6441&rank=2%7E31&source=see_link&search=amaurosis+fugax&utdPopup=true
— Shilpa
Agree with above. As Shilpa stated, the symptoms develop with edema associated with dying of the cyst. CT is a poor test for identifying the cysts, and most likely the MRI that was obtained after admission showed many lesions. I would ask the Neurosurgeon about giving steroids for the edema and a keppra load as mentioned above, to address her risk of seizure. My only other thoughts were to think of whether this lesion could be anything else. As mentioned, TB, CMV and Toxo are possibilities. And the lesion is calcified and not ring-enhancing, so likely not an abscess. But I would still think about risk factors for a brain abscess: any recent head trauma, recent dental procedures, murmur on exam, prolonged fever.